Following my last post and replies to it I have written a longer piece, which is pasted in below. Hope this helps and answers the questions.

Spino-Cerebellum Ataxia type 6

Brain training for speech problems

Introduction

I have SCA type 6, late onset. I am currently 65 and noticed the first effects of this about 7 years ago. I doubt if the brain cell loss in my cerebellum is likely to grow back, but we know less about the brain than any other part of the body. New discoveries are happening now with the onwards march of scanning techniques and the use of DNA and stem cells, so let's never say never.

My background

For the last 25 years I have been either a physical therapist and/or a psychotherapist. I have learnt a lot about the body, the mind and the patterns and strategies we all run internally, which manifest themselves as behaviours, beliefs, thoughts and actions.

This may or may not be true, however.....

I have made some statements below which may or may not be entirely accurate or 'true'. However if you behave as though they are true you may find a way to improve your lot and your enjoyment of life. I hope this happens and for myself I am constantly seeking ways to mitigate the effect of SCA. The following is offered in a sincere hope it is helpful.

Before you read on

Make yourself your favourite beverage and be comfy. This goes on a bit!

Plasticity is our friend

The brain has a unique ability called 'plasticity', which enables it to create new connections between neurons, when an existing connection becomes destroyed or corrupted in some way. People who have suffered a stroke, and lost the ability to speak, can make use of this, and often do without knowing that is what they are doing, to regain speech. Equally brain injury through blunt force trauma (car crash) can also destroy parts of the brain, and the brain is able to forge new pathways to recover what was lost. This often doesn't hold true in the same way for memory, as memories are usually located in a definite location in the brain and loss of the pathway can mean some memories can be lost. Though new pathways from a different direction can also turn up long lost memories.

The cerebellum, bless it

In spino-cerebellum ataxia the bit of brain being affected is the cerebellum, at the back of the head near to the brain stem. In evolutionary terms this is often considered the oldest part of the brain and is sometimes referred to as our reptilian brain. It's responses to outside stimuli are instinctive and faster than the rest of our brain can think. It plays a vital role in protecting us from danger. As an example on a couple of occasions when making a hot drink, I have moved my hand too close to the hot kettle. My automatic and instinctive response is to pull my hand away sharply. On doing this my hand caught the mug of hot liquid and threw it over my trousers!! not the original plan but a consequence of instinctive action beyond my conscious control. With time to think I would not have reacted so fast, but could also have burnt my hand. So sometimes our brain's reactions are not in our best interest.

The cerebellum also controls much of our movement and people like us with SCA know how this feels when it sends wobbly messages to our muscles. We think we are heading in one direction but seem to be going there in a roundabout route! The cerebellum does not know in advance the effect it will have as it has learnt over our lifetime how to propel us around. It is sending out the same signals, except because of the DNA corruption the messages are no longer the same. The same thing happens when we try to speak. The signal to move muscles in the jaw and mouth are sent out corrupted. Slurring occurs. We could describe this as 'On the inside I am perfectly clear, but when the words leave my mouth they don't make the sense they did when I said them on the inside'. So as I read this to myself all the words are perfectly clear to me, but if I read it out loud my tongue may trip and splutter over some of the words. (I'm aware of a tightening of the tension in my jaw and somedays this tension will give me a headache). The difference between SCA and a stroke is that we haven't lost a part of our brain, it's just not functioning right. It will however continue to function, do it's best and resist our efforts to circumvent it or find other ways to speak.

Automation – the key to human evolution

One of the other great abilities the human brain posseses is to take actions or thoughts we do or have regularly and automate them. It begins to do this before we are born and sets up breathing, circulation, digestion and other bodily systems to function without interference from the human who owns the body. Many people operate on this auto pilot system very succesfully for their entire life. Some people change the effectiveness of the system by interfering with it. For example with poor posture and poor breathing habits. This can be caused by life or by choice. Whichever, the 'system' works round it and is always attempting to return the body to the right place. (stasis).

This automation is so effective that we adopt it for other outside things. For example when you first learnt to talk, walk, run, read, write, dress yourself, etc, etc you began by copying or modelling others and then once you had worked out pretty much the way to do it your brain took over and you now do most of these things automatically, even unthinkingly. (Even slurred speech).

A brief practical demonstration. Please join in

Just to demonstrate the power of these unconscious habits I want you to lace your fingers together, with the palms of your hands facing towards you. One index finger will now be above or on top of the other. Note which one, left or right. Now take your fingers apart and put them back again with the other index finger on top. This may have been easy, or you may not have managed it at all. I have seen both results and frankly it doesn't matter if it was easy or hard, but you will probably have experienced it as different from your usual way. We have a definite way of crossing our arms too. Just automatic habits taken over by our brain to leave us room to think about the important things in life. E.g. Should we set up a colony on the moon? - Who's next to die in Eastenders? Stuff like that.

The power of forming habits

The point of talking about the power of habits is they are very powerful. Once we have given over this power to our brain it will continue to run the programme (software) even when it no longer helps us. It is very hard to give up an ingrained habit. (Smoking, drinking, nail biting, overeating, silly laugh, etc). In this sense those of us with SCA are at the mercy of our cerebellum and it's habits, which, due to the interference of our mutant DNA, don't come out as they used to.

Learning new habits

As it is hard to give up old habits the best way forward is to cultivate and learn new habits. In the hand clasping example above, if you want to be able to clasp your hands with either finger on top you need to practice the 'uncomfortable' way at least 20 times a day for a week. At the end of this time I bet you won't remember which way was 'right' and which way was 'wrong', as they are both 'right' now.

Effort required

Humans, and the human brain are lazy. Once they have found an effective way to do stuff they stop there. No need to explore other ways, what we have works okay. So if, like me you have some mental habits which no longer serve you, a way must be devised to learn new ways, which will serve you better. This sounds like it might be hard work. When we are already struggling with 'normal' stuff like walking straight and talking clearly, asking for something else may seem daunting.

Make learning fun

Think of a hobby or interest you may have now or used to have. As you learnt new things I bet you felt better and better about your hobby. As you mastered new skills, techniques or added to your knowledge, so the hobby gave you more pleasure and satisfaction. Let's make learning to talk in a new way a fun activity. (more below).

Harness the power of habits

Regular practice of anything leads to mastery, or at least a passing competence.

How do you know something has become a habit? When I first learnt to cut wood I often found the straight cut I thought I had made was only straight on the top of the wood. The bit I couldn't see was not straight. Over time I mastered simple cuts and now do it without thinking. Just occasionally I do focus on what I'm doing. When this happens the cut ceases to be straight as my mind gets in the way of the auto pilot and causes my hand to tense and make the saw wobble.

My wife is a very competent seamstress, and threads needles with ease, until she focusses on doing this, when she sticks the needle into her finger. You can think of examples of your own when you have interfered with a perfectly good auto system and got it wrong as a result.

Pulling this together

Make it fun

Make it regular

Form new habits

Use technology to help you

Share with others

Two sides of talking – physical – mental

The glitch in our slurred speech is caused by a physical impairment of the signal from our cerebellum to our speaking apparatus. Hence the disconnect between the clear speech inside our head and the sometimes garbled mess that comes out. (I've always spoken rubbish, but now I skpeo burirsh?)

Physical - Begin again

When we learnt out first language we did so by listening to others, watching their faces and playing with the sounds for ourselves. I remember listening outside the door of my son's room when he was learning to speak, aged 18 months. He would spend hours trying out different sounds and playing with the effects of movement of his tongue, jaw etc. Once we have 'got' the basics, guess what, our auto system takes over. The human voice is a great instrument, but once we have grooved our way of speaking, this is set and we don't usually change it. We can change it, we just usually don't bother. A public example was Margaret Thatcher. She had voice training to deepen her voice, so she had more authority. Talented mimics spend hours changing the shapes they make with their mouth, teeth and tongue to replicate the way others speak. Most of us assume our voice is stuck how it is and can't be changed, because of the power of habit and the laziness of our brain to do something else when the voice we have seems to be working okay. (Do you ever wish your voice sounded different? Deeper, sexier?)

Have you ever tried to learn a foreign or second language?

The very best way is by immersion in the language and culture. You not only learn the words, you learn the intonation, you learn the jaw, throat, tongue, lip movements, you learn the body language and subtle signs not available in books. You are fully immersed in a learning and modelling environment. You will also pick up the accent, if any, of the region where you learn. Ever heard a frenchman speaking english in a geordie accent? It's priceless!

So to begin again with your native language requires learning some of those basic mechanical actions, which until recently worked on auto-pilot. This time round you have some advantages, as you already have a good vocabulary, a grasp of grammar and an understanding of the body language which works in english. (assuming that's the language you want to work on).

Doing this for yourself – mental approach

A small diversion. Deaf people talk in a different way from hearing people. Because they only have the inside sound to go on. Hearing people can hear themselves from the outside as they talk, and this teaches us to speak more clearly than our short hand thoughts inside. Better speech is usually slower speech, though loud and fast seems to be the only way to get heard sometimes.

Set up your own feedback loop

I recently found an app on my iphone, or rather in the app store, called 'Feedback recorder'. Like many apps the basic model is free, with a small upgrade charge for enhanced functionality. I'm sure an android version exists, have a look if that's what you need.

Using my iphone microphone and my iphone earplugs I can speak out loud and simultaneously hear my voice from outside my head right in my ears. This gives me real time feedback on how I'm sounding. I can vary the speed of my voice, deepen the tone, sound like mickey mouse or deputy dawg, increase or decrease the volume, etc etc.

While this sounds like a bit of harmless fun, it's actually a way to retrain your 'speaking gear' to make the sounds you want heard. (Make me a cup of tea!)

Find a quiet place to do this, the microphone picks up all noises, and find something to read out loud. Peferably with long words and complex sentences. Saying the months of the year or the days of the week will not give you enough information to really make a difference to your mouthpiece.

For real fun google 'tongue twisters' and print out some useful ditties to challenge your brain mouth co-ordination. Look up vocal warm up exercises. These will include notes to sing and things to do to relax, stretch and loosen or tighten your jaw muscles. Also ways to move your tongue and change it's position for less stumbling.

And then – an internal feedback loop

Once you have spent some time playing with this external feedback system it's time to consider setting up an internal system in your own head.

Let me remind you of the amazing gift of brain plasticity.

Step one - Choose a place in your head, I suggest to one side and where you can easily touch it.

Step two - While speaking and listening to your voice using the external feedback system, touch the chosen part of your head and imagine that part of your brain beginning to learn how to provide you with feedback as you speak. Imagine a microphone in your ear picking up your speech and relaying it in real time to your mouth. You may have your own images of how this works for you, they will be better for you than mine.

Let me know how you get on

You may find even more useful resources. Please share.

I know this hasn't stopped you falling over. Let's look at that another time, but if you can keep communicating and don't get imprisoned in your head by bars of incomprehension, then life still has much to offer.

thank you SO much for this post which I read with great interest. I myself am a senior nurse who has helped many people with strokes rehabilitate and am trying now to work with myself following this diagnosis.

i hàve been constantly repeating the months of the year and the days of the week and it has helped a bit but will now concentrate on your advice.

good luck with all your endeavours and like you I try to stay positive.

Hi Nigel interesting read i had a stroke with right sided facial palsey,until 8 months after my stroke i did not realize my speech was slurred in my ears i sounded like i always had.then i met another stroke surviver who had the same problem and then realized my speech was slurred, i use cortana on my phone and some days have to repeat myself many times until she understands me,i have found if i speak just a few words at a time rather than a full sentance it is better.

Reading this has just made so much sense to me! I have got very late on-set FA (in my 40's before any signs) but it has put into words what I have tried explaining to numerous people many times how it feels. I have more problems with balance, co-ordination and walking rather than speech. (although it does get difficult when I am in unknown company) I am seeing a neurophysiotherapist who is trying to get my posture back to 'normal' so that my walking is more balanced. All made more difficult by an old injury which left me with one leg 2cm shorter than the other so I have walked lopsided for over 30 years anyway so has become even worse with the ataxic way of walking! I shall use your examples of lacing fingers together or crossing your arms when I want someone to understand what I am having to do all the time. I hope your speech improves with using the app!

what a very informative post. Thanks. I only have speech problems when I'm really tired so far. But you're theory is exactly what my physiotherapist has explained to me. We have to keep repeating an action to relearn it, as much as our fatigue will allow. I can actually do more than I could two years ago. Hard work but worth it😊.

I will use the finger lacing and arm crossing to help people have a little idea of what our life is like!.

Thank you, Nigel for all the great information! I'm currently reading Norman Doidge, M.D.'s books, "The Brain's Way Of Healing" and "The Brain That Changes Itself", both about neuroplasticity. I notice if I challenge myself (although difficult), I do notice supple changes! So I will continue this! The key is making these challenges "fun"! My best to you...,;o)

Repetition is certainly the start point. I can see that tapping could indeed be helpful as it focuses the mind and reinforces the messages you want your mind/body to act on. I'm reading about using the mind to heal the mind and at present this is suggesting daily repititions. Getting into good habits is certainly the best way to live!

Research shows that perseverance brings rewards, so keep up the practice.

Thanks Nigel.At last some optimism.I have hadCA for some 6/7 years.The combination of smyptoms;tremor,no mobility,failing eyesight and rapdly worsening speech has left me feeling hopeless.Your post about plasticity of the brain has given me some hope.I'm in my mjd fifties and not ready for the scrap heap.

Dear Nigel, So glad you're enjoying the books on neuroplasicity! I am also and agree, it's up to us to fight this! My neurologist says there's been successful studies on rodents where they make new brain pathways by doings things over and over. The key to this is repetition, where you keep challenging yourself. For instance, I do a balance exercise daily at the ledge between my kitchen and family room (in case I have to grab on to prevent falling). I stand on one foot for as many seconds as I can, and then the other one. I keep trying to up the seconds. I look forward to hearing more from you...,;o)

I've started a website to put on all the ways I'm finding to fight back against my wibbly wobbly gene. It's at ataxiafightback.wordpress.com please have a look and let me know what else would be useful.